Management of Scoliosis in Patients With Loeys-Dietz Syndrome

Jarred A. Bressner, Gretchen L. MacCarrick, Harry C Dietz, Paul David Sponseller

Research output: Contribution to journalArticle

Abstract

BACKGROUND:: Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder. We sought to determine the incidence of scoliosis in patients with LDS, characterize the spectrum of spinal deformity, determine the results of bracing and surgery, and define surgical complications. METHODS:: Patients were selected from our institution’s database of 183 patients with LDS. Imaging measurements were performed for 141 patients whose records permitted spinal evaluation. Deformity changes and complications after intervention were recorded for patients who underwent bracing or surgery, and associations were tested using Student t tests (significance, P0.05). Nine patients, (mean age, 12±3 y), underwent 24 surgical procedures (16 growing rod procedures, 8 fusions). Mean curve corrections were 61% for growing rods and 73% for fusions. Associated blood loss for these procedures was 400 mL and 1293 mL, respectively, and normalized blood loss for fusion was 2.34 mL/kg/level. Fifteen of 24 surgical procedures involved complications (63%), including cerebrospinal fluid leaks (n=7) and blood loss >20% of estimated total blood volume (n=11). CONCLUSIONS:: Scoliosis was present in 62% of our sample of LDS patients. Bracing did not halt curves in 11 of 15 patients, whose curves progressed >5 degrees or to >50 degrees by completion of bracing. At latest follow-up, 47% of the braced patients had undergone surgery after prior bracing attempts. The high blood loss associated with these operations is believed to be related to vascular fragility in patients with LDS. LEVEL OF EVIDENCE:: Level IV—retrospective cohort study.

Original languageEnglish (US)
JournalJournal of Pediatric Orthopaedics
DOIs
StateAccepted/In press - Jul 2 2016

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Loeys-Dietz Syndrome
Scoliosis
Blood Volume
Connective Tissue
Blood Vessels

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

Cite this

Management of Scoliosis in Patients With Loeys-Dietz Syndrome. / Bressner, Jarred A.; MacCarrick, Gretchen L.; Dietz, Harry C; Sponseller, Paul David.

In: Journal of Pediatric Orthopaedics, 02.07.2016.

Research output: Contribution to journalArticle

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abstract = "BACKGROUND:: Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder. We sought to determine the incidence of scoliosis in patients with LDS, characterize the spectrum of spinal deformity, determine the results of bracing and surgery, and define surgical complications. METHODS:: Patients were selected from our institution’s database of 183 patients with LDS. Imaging measurements were performed for 141 patients whose records permitted spinal evaluation. Deformity changes and complications after intervention were recorded for patients who underwent bracing or surgery, and associations were tested using Student t tests (significance, P0.05). Nine patients, (mean age, 12±3 y), underwent 24 surgical procedures (16 growing rod procedures, 8 fusions). Mean curve corrections were 61{\%} for growing rods and 73{\%} for fusions. Associated blood loss for these procedures was 400 mL and 1293 mL, respectively, and normalized blood loss for fusion was 2.34 mL/kg/level. Fifteen of 24 surgical procedures involved complications (63{\%}), including cerebrospinal fluid leaks (n=7) and blood loss >20{\%} of estimated total blood volume (n=11). CONCLUSIONS:: Scoliosis was present in 62{\%} of our sample of LDS patients. Bracing did not halt curves in 11 of 15 patients, whose curves progressed >5 degrees or to >50 degrees by completion of bracing. At latest follow-up, 47{\%} of the braced patients had undergone surgery after prior bracing attempts. The high blood loss associated with these operations is believed to be related to vascular fragility in patients with LDS. LEVEL OF EVIDENCE:: Level IV—retrospective cohort study.",
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